Problems with Gas Transport

By:
Bryan Mae H. Degorio, BSN,
RN
Congenital Heart Diseases
2 CLASSIFICATIONS
C.Acyanotic type
- there is shunting of blood from the
left side of the heart to the
right side of the heart
- there is no abnormal connection
between pulmonary and
systemic circulation
- the connection within the heart
chambers forces blood to from
the arterial (left) to the
venous (right) side of the heart,
where it is reoxygenated
 - if left untreated, it will lead to heart
failure

- Signs of Left-sided Heart Failure:

a. Feeding difficulty - usually the first
sign because of easy fatigability
when sucking or feeding
b. Pallor
c. tachypnea or rapid breathing
- signs of Right-sided Heart Failure
a. Hepatomegaly, abdominal pain
b. Anorexia
c. jugular vein distention
d. diaphoresis
e. Edema - late sign
 - management:
1. digitalis- has a positive inotropic and
negative chronotropic
2. reduce cardiac work load
a. Complete bed rest
b. Orthopneic position
c. small frequent feeding
d. minimal handling
3. remove fluid accumulation
a. Diuretics d. weigh daily
b. Low sodium diet
c. I and O monitoring
 3. administer oxygen to improve tissue
perfusion
Ventricular Septal Defect
(VSD)
- there is abnormal opening between
the ventricles
 Pathophysiology

Right atrium into the Left and right

right ventricle pulmonary artery
Unoxygenated
ventricles via via the pulmonic
blood
tricuspid valve

Left and right Oxygenated lungs

pulmonary veins blood
Diminished
supply of
Left atrium into the Aorta via oxygenated
left ventricle via the aortic valve blood to the
Bicuspid valve systemic
RIGHT ATRIUM circulation
via SEPTAL
DEFECT

Reoxygenation of
blood to the lungs
 - manifestations:
2.Low harsh murmur heard throughout
the systole –
pathognomonic sign
3.Right ventricular hyperthropy
4.Bradycardia, dyspnea and slowing of
growth pattern
5.Prone to URTI and bacterial
endocarditis
 - Management:
2.Large septal defect- closure with the
use of plastic knitted dacron
patch in a cardiopulmonary
bypass through the right
atrium and the tricuspid
valve
3.Small septal defect- will close
spontaneously or with the
use of purse-string
approach
Patent Ductus Arteriosus
(PDA)
- failure of the fetal aorta and
pulmonary artery to close
- commonly associated to mother
with puerperial rubella
infection
 Pathophysiology

Right atrium into the Left and right

right ventricle pulmonary artery
Unoxygenated
ventricles via via the pulmonic
blood
tricuspid valve

Left and right Oxygenated lungs

pulmonary veins blood
PULMONARY
ARTERY via Reoxygenation
Left atrium into the
PATENT of blood to the
left ventricle via the
DUCUS lungs
Bicuspid valve
ARTERIOSUS
Diminished
Aorta via supply of
aortic valve oxygenated
blood to the
systemic
circulation
 - manifestations:
 Shunting of blood leading to pulmonary
hypertension and cardiomegaly
 Machinery like murmur heard
throughout the heartbeat in the left
2nd or 3rd intercostal area -
pathognomonic sign
 Prominent radial pulse in the newborn
 Limited growth and physical activity
 LEFT Ventricular enlargement on ECG
and Chest x-ray
 - Management:
2.Closure of the opening at the 1 to 4
years through ligation or division
3.Use of endomethacin (prostaglandin
inhibitor) is successful in closing a
patent ductus arteriosus

- Common complication:
1. Bacterial endocarditis due
streptococcal infection
Coarctation of the Aorta
- narrowing of the aorta within the
ductus arteriosus
 Pathophysiology

Right atrium into the Left and right

right ventricle pulmonary artery
Unoxygenated
ventricles via via the pulmonic
blood
tricuspid valve

Left and right Oxygenated lungs

pulmonary veins blood

Left atrium into the COARCTED

left ventricle via the AORTA via
Bicuspid valve AORTIC VALVE

Diminished
supply of
oxygenated
blood to the
systemic
circulation
 - manifestations:
2.Absence of femoral pulse-
pathognomonic sign
3.Increased systemic circulation
4.BP is higher in the upper extremities
due to peripheral vascular resistance
resulting to bounding radial and carotid
pulse, headache, dizziness and
epistaxis
5.Intermittent claudication, cold feet,
muscle spasm, weak, delayed or
absent pulse
6.Left ventricular hyperthropy
 - management:
2.Resection of the defect and
anastomosis of the end of the aorta or
enlargement of the constricted
section using a graft of prosthetic
material or a portion of left
subclavian artery ; surgery until 8-10
years old
3.Balloon angioplasty is a primary
intervention for COA is being
performed in some centers
Aortic Stenosis
- narrowing of the aortic valve
 Pathophysiology

Right atrium into the Left and right

right ventricle pulmonary artery
Unoxygenated
ventricles via via the pulmonic
blood
tricuspid valve

Left and right Oxygenated lungs

pulmonary veins blood

Left atrium into the NARROWED

left ventricle via the AORTA via
Bicuspid valve AORTIC VALVE

Diminished
supply of
oxygenated
blood to the
systemic
circulation
 - Manifestations:
2.Increased workload on the left
ventricle and the lowered
pressure in the aorta reduces
coronary artery flow
- management:
1. Valvotomy or balloon angioplasty or
division of the stenotic valve
B. Cyanotic type
- shunt from the right to left side of
the heart
- indicates an abnormality that
permits the systemic venous
return (unoxygenated
blood) to bypass the lungs and
enter the general
circulation directly
 - Characteristics of Cyanotic type
2.Abnormal connection between the
pulmonary and systemic circulation
3.Venous (unoxygenated) blood enters
systemic circulation
4.Polycythemia occurs as the body
compensate for inadequate supply
of oxygen
5.Squatting or Knee chest position is
preferred to lessen the venous return
by constricting the femoral vein
Tetralogy of Fallot
- has four associated defects:
a. Pulmonary vein stenosis
b. Ventricular septal defect
c. Overriding of the aorta- receiving
blood from both ventricle or
an aorta arising from the
right ventricle
d. Right ventricular hyperthropy
 Pathophysiology
PULMONARY
Right atrium into the ARTERY via
Unoxygenated right ventricle STENOSED
blood ventricles via PULMONARY
tricuspid VALVE

Decrease blood
SHUNTING of INCREASED delivered to the
UNOXYGENATED PRESSURE ON THE lungs
BLOOD to the RIGHT VENTRICLE
OVERRIDING
AORTA
SHUNTING of
RIGHT UNOXYGENATED
VENTRICULER BLOOD through the
Diminished HYPERTHROPHY SEPTAL DEFECT going
supply of to the LEFT
oxygenated VENTRICLE
blood to the
systemic
circulation
 - manifestations:
2.Cyanosis – BLUE BABY
3.Clubbing of finger and toes
4.Growth retardation
5.Exertional dyspnea relieved
SQUATTING- due to rapid saturation
return
6.Polycythemia
7.Right ventricular hyperthropy – x-ray
result
8.“Boot shaped” configuration of the
heart
 - management:
2.Blalock-Taussig procedure – subclavian
artery to pulmonary artery
anastomosis
3.Complete repair – closure of the
ventricular septal defect and
resection of the infundibular
stenosis
Transposition of Great Vessels
- aorta arise from the right ventricle
and the pulmonary artery
arises from the left ventricle
- incompatible
for life
 Pathophysiology

Right atrium into the

right ventricle UNOXYGENATED
Unoxygenated BLOOD going the
blood ventricles via
tricuspid AORTA

lungs Oxygenated Unoxygenated

blood blood to the
systemic
circulation
Left and right
pulmonary veins

LEFT and Left atrium into the

RIGHT left ventricle via the
PULMONERY Bicuspid valve
ARTERY
 - Management
B.Palliative Management
- performed to prevent
pulmonary vascular
resistance and CHF until the
child id able to tolerate complete repair
1. Rashkind procedure – enlargement
of an existing atrial septal
defect by pulling a balloon
through the defect during cardiac
catheterization
2. Pulmonary artery banding – to
decrease the blood flow to the
lungs and increase shunting of
oxygenated blood
intraventricularly into the aorta
3. Pharmacologic dilation of PDA with
the use of prostaglandin
4. Blalock-hanton – surgical creation
ASD
B. Complete Repair:
1. Rastelli’s procedure - closure of
VSD
2. Mustard or Seening Procedure –
removing and entire
atrial septum and creating
a new atrial septum from
existing pericardium
3. Jatene Operation – transposing the
great vessel to correct the
anatomic placement with
reimplantation of the coronary
arteries
Tricuspid Atresia
- absence of tricuspid valve
- incompatible for life unless with
ASD, VSD or PDA
 - management:
2.Palliative treatment procedure – same
as with TOF
3.Complete repair
a. Modified Fontan procedure –
conversion of the right atrium
into an outlet for pulmonary
artery
Truncus Arteriosus
- single great vessel arising from
base of the heart, serving as
pulmonary and aorta
 - manifestations:
2.Systolic murmur with a loud second
heart sound that is not soft prosthetic
valve split

- Management:
5.Palliative – banding of pulmonary
arteries to decrease blood flow to
the lungs
6.Complete repair – Rastelli’s procedure
wherein the existing
pulmonary arteries from the
aorta and attaching them to the
Sickle Cell Anemia
- is a severe, chronic, hemolytic
anemia occurring in
persons who are
homozygous for the sickle gene
- is a group of hemoglobinopthies, in
which the normal adult
haemoglobin A ( HbA) is
partly or completely replaced
by abnormal sickle hemoglobin ( HbS )
- statistics:
-With high incidence among
BLACKS
- 8% among Blacks
 Sickle Shape
Cell

Entanglement and
Enmeshing of rigid sickle
shape cell

RBC
OBSTRUCTION
DESTRUCTION

1. Stasis with enlargement

2. Infarction with ischemia and

destruction
3. Replacement with fibrous
tissue
Clinical manifestations:
2.Signs of Anemia
a. Hemoglobin – 6g/dl to 9g/dl
b. Loss of appetite
c. Paleness and weakness
d. Fever and irritability
e. Jaundice due to increase
hemolysis resulting to
hemosiderosis
2. Crises Manifestations:
crises- acute symptoms of the
disease occurring
during the period of
exacerbation
- predisposing factor for
Crises:
a. Dehydration f. Cold
exposure
b. Infection g. hypoxia
c. trauma h. acidosis
d. strenuous physical exercises
e. Extreme fatigue
 - Types of Crises
A. Vaso-occlusive disease
- preferably called “ painful
episodes”
- extremities:
a. Bony destructions – related
to
hyperplasia of the
marrow
b. Bone pain and swelling
joint
c. dactylitis (hand-foot
syndrome)
- aseptic infarction
 3. spleen
a. Abdominal pain
b. Spleenomegaly- due to RBC
destruction
c. increase the risk of infection
d. spleen becomes atrophied
and fibrotic
4. cerebral occlusion
a. Stroke, hemiplegia
b. Retinal damage leading to
blindness
c. seizure
 5. pulmonary infarction
6. altered renal function- enuresis
and hematuria
7. impaired liver function
8. Priapism- abnormal, recurrent,
prolonged and
painful penile
erection
B. Sequestration crises
- due to large amount of blood
pooled in the spleen
 1. Spleen becomes massively
enlarge
2. Great decrease in RBC mass
3. signs of circulatory collapse
4. hepatomegaly
C. Aplastic crises
1. diminished RBC production
resulting to profound
anemia
Diagnostic Procedure:
2.Sickle cell prep (sickling test)
- done by finger or heel stick
- oxygen is removed from the
dropped blood
- the blood is observed under the
microscope for
sickle shape
- test done to distinguish person
with sickle cell trait and
disease
2. Sickle turbidity test (sickledex)
- done by finger stick
- a small amount of blood is
placed in the solution
containing chemical
reducing agent
- sickle hemoglobin is indicated if
the solutions turn turbid
3. Hemoglobin electrophoresis
- requires venipuncture
- is an accurate, rapid and specific
test for detecting
homozygous and
heterozygous form of the disease
Management:
2.Bed rest to minimize the energy
expenditures
3.Hydration through oral and IV
therapy
4.Electrolyte replacement
5.Analgesic for severe pain associated
with vaso-occlusion
6.Blood replacement to treat anemia
and to reduce the viscosity of the
sickled blood
7.Antibiotics to treat any infection
 - administration of pneumococcal
and meningococcal
vaccine and hepa B vaccine